Oral Status of a Pediatric Patient with Merosine Negative Congenital Muscular Dystrophy

Abstract

Merosine Deficient Congenital Muscular Dystrophy (MDCMN) is a rare subtype of Muscular Dystrophy and it is defined as generalized hypotonic muscular syndrome. This case report aimed to describe the oral status of a six-yearold girl with MDCMN. The family’s and child’s chief complaint was bad breath. The child, who presented normal intelligence, had physical disability with mobility impairment and so, used wheelchair. Her feeding was done mostly through gastric feed tube, however, the child used to eat soft food, liquids and some candies by mouth. The extraoral examination showed facial hypotonic muscles, inadequate labial sealing and bimaxillary discrepancies. While the intraoral exam revealed mixed dentition with infraocclusion of both second primary molars and class III molar occlusion. Although the child was caries-free, her oral hygiene was deficient and dental calculus were observed on the lower teeth. In addition, the intrusion of the right upper primary canine. Radiographic findings included agenesis of the lower second premolar bud. Mechanical debridation, prophylaxis and fluoride application were performed. Besides, both mother and child were instructed and motivated in relation to oral hygiene (tongue scrapping) and diet, in order to minimize halitosis. Muscular Dystrophy brings to the patients several complications, including oral manifestations. Thus, oral health plays an important role and the dentist needs to pay attention, in order to contribute the improvement of the quality of life of these patients.